Lichen myxedematosus is a rare, chronic, progressive disease of unknown etiology characterized histologically by mucin deposition in the upper one half of the dermis and is in many cases associated with a serum paraprotein. We report the third case of lichen myxedematosus which has been treated with oral cyclophosphamide. Our case, as one of those previously treated, lacked the characteristic paraprotein. Therapeutic response consisted of improvement, but not complete resolution of the skin findings.